(S)-carnitine

Three steps

• L-kynurenineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. L-kynurenine + H2O = anthranilate + L-alanine + H(+)

  Catalysed by CPT1A, CPT1C, CPT1B, KYNUcytosol, mitochondrial matrixOne of 5 routes

• a 1-acyl-sn-glycero-3-phosphateReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. sn-glycerol 3-phosphate + an acyl-CoA = a 1-acyl-sn-glycero-3-phosphate + CoA

  Catalysed by CPT1A, CPT1C, CPT1B, ASAH1 and 7 morecytosol, Golgi apparatus

• leukotriene C4Reach from here →
  1. Carnitine O-Palmitoyltransferase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. leukotriene C4 hydrolysis

  Catalysed by CPT1A, CPT1C, CPT1B, GLYAT and 2 morecytosol, mitochondrial matrixOne of 10 routes

• taurocyamineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)
  3. taurine + L-arginine = taurocyamine + L-ornithine

  Catalysed by CPT1A, CPT1C, CPT1B, BAAT and 1 morecytosolOne of 2 routes

• L-cysteateReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)
  3. L-cysteate + H(+) = taurine + CO2

  Catalysed by CPT1A, CPT1C, CPT1B, BAAT and 2 morecytosol

• N(1)-acetylspermineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. N(1)-acetylspermine + O2 + H2O = 3-acetamidopropanal + spermidine + H2O2

  Catalysed by CPT1A, CPT1C, CPT1B, SAT1 and 2 morecytosol

• 3-hydroxy-L-kynurenineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. 3-hydroxy-L-kynurenine + H2O = 3-hydroxyanthranilate + L-alanine + H(+)

  Catalysed by CPT1A, CPT1C, CPT1B, KYNUcytosol, mitochondrial matrix

• gamma-L-glutamyl-L-cysteineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. L-glutamate:L-cysteine gamma-ligase (ADP-forming)

  Catalysed by CPT1A, CPT1C, CPT1B, NAGS and 2 morecytosol, mitochondrial matrixOne of 2 routes

• 6-amino-2-oxohexanoateReach from here →
  1. Carnitine Transport by Atb0
  2. transport of chloride and lysine (cytosol to extracellular)
  3. L-lysine:oxygen 2-oxidoreductase (deaminating)

  Catalysed by SLC6A14extracellular space, cytosol, peroxisome

• L-homocysteineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. L-homocysteine + L-serine = L,L-cystathionine + H2O

  Catalysed by CPT1A, CPT1C, CPT1B, NAGS and 1 morecytosol, mitochondrial matrixOne of 2 routes

• carbamoyl phosphateReach from here →
  1. Carnitine Transport by Atb0
  2. Transport of L-Glutamine by Atb0 Transporter
  3. HCO3-:L-glutamine amido-ligase (ADP-forming, carbamate-phosphorylating)

  Catalysed by SLC6A14, CADextracellular space, cytosolOne of 2 routes

• adenosine 5'-phosphosulfateReach from here →
  1. Carnitine Transport by Atb0
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. ATP:sulfate adenylyltransferase

  Catalysed by SLC6A14, SLC26A6, PAPSS1, PAPSS2extracellular space, cytosolOne of 4 routes

• N,N-dimethylglycineReach from here →
  1. Carnitine Transport by Atb0
  2. transport of betaine and chloride (cytosol to extracellular)
  3. L-homocysteine + glycine betaine = N,N-dimethylglycine + L-methionine

  Catalysed by SLC6A14, SLC6A12, BHMTextracellular space, cytosol

• S-methyl-L-methionineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by CPT1A, CPT1C, CPT1B, NAGS and 1 morecytosol, mitochondrial matrix

• a 1-acyl-2-(9Z)-octadecenoyl-sn-glycero-3-phosphocholineReach from here →
  1. Utilized Transport
  2. (9Z)-octadecenoate + ATP + CoA = (9Z)-octadecenoyl-CoA + AMP + diphosphate
  3. a 1-acyl-sn-glycero-3-phosphocholine + (9Z)-octadecenoyl-CoA = a 1-acyl-2-(9Z)-octadecenoyl-sn-glycero-3-phosphocholine + CoA

  Catalysed by SLC27A2, ACSL3, ACSL1, ACSBG2 and 5 moreendoplasmic reticulum, cytosol

• glutathione disulfideReach from here →
  1. Utilized Transport
  2. Arachidonate:oxygen 5-oxidoreductase
  3. Glutathione: 5-HPETE oxidoreductase

  Catalysed by ALOX5, GPX5, GPX1, GPX2 and 6 moreendoplasmic reticulum, cytosolOne of 25 routes

• 3-oxosphinganineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. Palmitate:CoA ligase (AMP-forming)
  3. Palmitoyl-CoA:L-serine C-palmitoyltransferase (decarboxylating)

  Catalysed by CPT1A, CPT1C, CPT1B, SLC27A2 and 10 morecytosolOne of 2 routes

• taurolithocholate 3-sulfateReach from here →
  1. Utilized Transport
  2. transport of arachidonate (cytosol to extracellular)
  3. transport of S-glutathionyl-2-4-dinitrobenzene (cytosol to extracellular)

  Catalysed by SLCO1B1, SLCO1A2, SLCO1B3endoplasmic reticulum, cytosol, extracellular spaceOne of 4 routes

• 1-hexadecanoylglycerone 3-phosphateReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. Palmitate:CoA ligase (AMP-forming)
  3. glyceronephosphate O-acyltransferase (DHAP)

  Catalysed by CPT1A, CPT1C, CPT1B, SLC27A2 and 8 morecytosol, peroxisomeOne of 2 routes

• L-tyrosineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine

  Catalysed by CPT1A, CPT1C, CPT1B, GLYAT and 3 morecytosol, mitochondrial matrixOne of 2 routes

• N(1)-acetylspermidineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. spermidine + acetyl-CoA = N(1)-acetylspermidine + CoA + H(+)

  Catalysed by CPT1A, CPT1C, CPT1B, SAT1 and 1 morecytosol

• N(omega),N(omega)-dimethyl-L-arginineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine

  Catalysed by CPT1A, CPT1C, CPT1B, GLYAT and 3 morecytosol, mitochondrial matrixOne of 4 routes

• 3-aminopropanalReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. diamine N-acetyltransferase (N1-acetylspermine)
  3. spermidine:oxygen oxidoreductase (spermidine-forming)

  Catalysed by CPT1A, CPT1C, CPT1B, SAT1 and 2 morecytosol

• trans-4-hydroxy-L-prolineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. trans-4-hydroxy-L-proline(out) + L-alanine(in) + Na(+)(out) = trans-4-hydroxy-L-proline(in) + L-alanine(out) + Na(+)(in)

  Catalysed by CPT1A, CPT1C, CPT1B, SLC1A4cytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• histamineReach from here →
  1. Utilized Transport
  2. transport of arachidonate (cytosol to extracellular)
  3. transport of histamine (cytosol to extracellular)

  Catalysed by SLCO1B1, SLCO1A2, SLCO1B3endoplasmic reticulum, cytosol, extracellular spaceOne of 5 routes

• sn-glycero-3-phospho-1D-myo-inositolReach from here →
  1. Utilized Transport
  2. 1-(9Z-octadecenoyl)-sn-glycero-3-phospho-(1D-myo-inositol) + H2O = sn-glycero-3-phospho-1D-myo-inositol + (9Z)-octadecenoate + H(+)
  3. 1-(9Z-octadecenoyl)-sn-glycero-3-phospho-(1D-myo-inositol) + H2O = sn-glycero-3-phospho-1D-myo-inositol + (9Z)-octadecenoate + H(+)

  Catalysed by ABHD12endoplasmic reticulum, cytosol

• L-thyroxineReach from here →
  1. Utilized Transport
  2. transport of arachidonate (cytosol to extracellular)
  3. transport of S-glutathionyl-2-4-dinitrobenzene (cytosol to extracellular)

  Catalysed by SLCO1B1, SLCO1A2, SLCO1B3endoplasmic reticulum, cytosol, extracellular spaceOne of 6 routes

• 5-guanidino-2-oxopentanoateReach from here →
  1. Carnitine Transport by Atb0
  2. transport of arginine and chloride (cytosol to extracellular)
  3. L-arginine + O2 + H2O = 5-guanidino-2-oxopentanoate + H2O2 + NH4(+)

  Catalysed by SLC6A14, IL4I1extracellular space, cytosol

• N(8)-acetylspermidineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. N8-acetylspermidine to spermidine conversion

  Catalysed by CPT1A, CPT1C, CPT1B, SAT1 and 1 morecytosolOne of 3 routes

• N(1),N(12)-diacetylspermineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. N1,N12-diacetylspermine to N1-acetylspermine conversion
  3. N1,N12-diacetylspermine to N1-acetylspermine conversion

  Catalysed by CPT1A, CPT1C, CPT1BcytosolOne of 2 routes

• (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoateReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. long-chain-fatty-acid-CoA ligase (EPA)
  3. (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoA hydrolase

  Catalysed by CPT1A, CPT1C, CPT1B, SLC27A2 and 10 morecytosolOne of 14 routes

• S-sulfanyl-L-cysteineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. 2 L-cysteine = S-sulfanyl-L-cysteine + L-alanine

  Catalysed by CPT1A, CPT1C, CPT1B, NAGS and 1 morecytosol, mitochondrial matrix

• a 1,2-diacyl-sn-glycero-3-phosphateReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 1-acyl-sn-glycero-3-phosphate + an acyl-CoA = a 1,2-diacyl-sn-glycero-3-phosphate + CoA

  Catalysed by CPT1A, CPT1C, CPT1B, ASAH1 and 16 morecytosol, Golgi apparatus

• N-formyl-L-kynurenineReach from here →
  1. Carnitine Transport by Atb0
  2. Transport of L-Tryptophan by Atb0 Transporter
  3. L-tryptophan + O2 = N-formyl-L-kynurenine

  Catalysed by SLC6A14, IDO1, TDO2, IDO2extracellular space, cytosol

• (3S)-citramalyl-CoAReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. Itaconate:CoA ligase (GDP-forming)
  3. (3S)-citramalyl-CoA = itaconyl-CoA + H2O

  Catalysed by CPT1A, CPT1C, CPT1B, SUCLG1 and 3 morecytosol, mitochondrial matrix

• 5-phospho-beta-D-ribosylamineReach from here →
  1. Carnitine Transport by Atb0
  2. Transport of L-Glutamine by Atb0 Transporter
  3. phosphoribosyl pyrophosphate amidotransferase (glutamine)

  Catalysed by SLC6A14, PPATextracellular space, cytosolOne of 2 routes

• phosphoenolpyruvateReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. ATP citrate lyase (citrate)
  3. oxaloacetate + GTP = phosphoenolpyruvate + GDP + CO2

  Catalysed by CPT1A, CPT1C, CPT1B, ACLY and 2 morecytosol

• D-glucosamine 6-phosphateReach from here →
  1. Carnitine Transport by Atb0
  2. Transport of L-Glutamine by Atb0 Transporter
  3. D-fructose 6-phosphate + L-glutamine = D-glucosamine 6-phosphate + L-glutamate

  Catalysed by SLC6A14, GFPT2, GFPT1extracellular space, cytosol

• L-methionine (S)-S-oxideReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (S)-S-oxide + [thioredoxin]-dithiol

  Catalysed by CPT1A, CPT1C, CPT1B, NAGS and 1 morecytosol, mitochondrial matrix

• L-methionine (R)-S-oxideReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (R)-S-oxide + [thioredoxin]-dithiol

  Catalysed by CPT1A, CPT1C, CPT1B, NAGS and 3 morecytosol, mitochondrial matrix

• S-sulfanylglutathioneReach from here →
  1. Utilized Transport
  2. transport of arachidonate and GSH (cytosol to extracellular)
  3. Sulfide:glutathione,quinone oxidoreductase

  Catalysed by SQORendoplasmic reticulum, cytosol, extracellular space, mitochondrial matrix

• N-[(R)-4-phosphopantothenoyl]-L-cysteineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. (R)-4'-phosphopantothenate:L-cysteine ligase

  Catalysed by CPT1A, CPT1C, CPT1B, NAGS and 1 morecytosol, mitochondrial matrixOne of 4 routes

• monodehydro-L-ascorbate radicalReach from here →
  1. Utilized Transport
  2. arachidonate to 8-peroxy-(5Z,9E,11Z,14Z)-eicosatetraenoate conversion
  3. 8-peroxy-(5Z,9E,11Z,14Z)... to 5,9-cyclo-6,8-cycloperoxy-12... conversion

  No catalyst recordedendoplasmic reticulum, cytosol

• 3-iodo-L-tyrosineReach from here →
  1. Carnitine Transport by Atb0
  2. Transport of L-Tyrosine by Atb0 Transporter
  3. thyroid peroxidase (iodine)

  Catalysed by SLC6A14, TPOextracellular space, cytosolOne of 2 routes

• dopamineReach from here →
  1. Utilized Transport
  2. transport of arachidonate (cytosol to extracellular)
  3. transport of dopamine (cytosol to extracellular)

  Catalysed by SLCO1B1, SLCO1A2, SLCO1B3endoplasmic reticulum, cytosol, extracellular spaceOne of 4 routes

• 1-acyl-2-(9Z,12Z)-octadecadienoyl-sn-glycero-3-phosphocholineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
  3. (9Z,12Z)-octadecadienoyl-CoA + a 1-acyl-sn-glycero-3-phosphocholine = 1-acyl-2-(9Z,12Z)-octadecadienoyl-sn-glycero-3-phosphocholine + CoA

  Catalysed by CPT1A, CPT1C, CPT1B, SLC27A2 and 8 morecytosol

• glycolithocholate 3-sulfateReach from here →
  1. Utilized Transport
  2. transport of arachidonate (cytosol to extracellular)
  3. transport of S-glutathionyl-2-4-dinitrobenzene (cytosol to extracellular)

  Catalysed by SLCO1B1, SLCO1A2, SLCO1B3endoplasmic reticulum, cytosol, extracellular spaceOne of 4 routes

• a 2,3-dehydroacyl-CoAReach from here →
  1. Utilized Transport
  2. (5Z,8Z,11Z,14Z)-eicosatetraenoate + AH2 + 2 O2 = prostaglandin H2 + A + H2O
  3. a 2,3-saturated acyl-CoA + A = a 2,3-dehydroacyl-CoA + AH2

  Catalysed by PTGS1, PTGS2, ACAD10endoplasmic reticulum, cytosol

• L-prolineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. L-proline(out) + L-alanine(in) + Na(+)(out) = L-proline(in) + L-alanine(out) + Na(+)(in)

  Catalysed by CPT1A, CPT1C, CPT1B, SLC1A4cytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• estrone 3-sulfateReach from here →
  1. transport of butyryl-CoA and L-carnitine (cytosol to mitochondria)
  2. butanoyl-CoA + H2O = butanoate + CoA + H(+)
  3. butanoate(out) + estrone 3-sulfate(in) = butanoate(in) + estrone 3-sulfate(out)

  Catalysed by SLC33A1, ESCO2, OXSM, ACOT8 and 6 moremitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 5 routes